Abstract
Currently, the physiological mechanisms underlying auditory neuropathy are unclear, and there are likely to be multiple sites of lesion. A better understanding of the disruption in individual cases may lead to more effective management and device selection. Frequency-specifi c round-window electrocochleography (ECochG) waveforms were used to assess local hair cell, dendritic, and axonal currents generated within the cochlea in 15 subjects with auditory neuropathy (16 ears). These results were compared with electrically evoked auditory brainstem response (EABR) measured after cochlear implantation. The results of this study demonstrate that predominantly two patterns of ECochG waveforms can be identifi ed: (i) a prolonged latency of the hair cell summating potential (SP) waveform with or without residual CAP activity and (ii) a normal latency SP, typically followed by a dendritic potential (DP). We show that seven of eight subjects with a prolonged SP showed a normal EABR waveform, consistent with a presynaptic lesion, whereas six of seven subjects with a normal latency SP showed poor morphology or absent EABR waveforms, consistent with a postsynaptic lesion. We suggest that a presynaptic and postsynaptic type of auditory neuropathy exist, which may have implications for the fi tting of cochlear implants.
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References
Starr A, Picton T, Sininger Y, et al (1996) Auditory neuropathy. Brain 119:741–753
Rance G (2005) Auditory neuropathy/dys-synchrony and its perceptual consequences. Trends Amplif 9:1–43
Buss E, Labadie RF, Brown CJ, et al (2002) Outcome of cochlear implantation in pediatric auditory neuropathy. Otol Neurotol 23:328–332
Rance G, Cone-Wesson B, Wunderlich J, et al (2002) Speech perception and cortical event related potentials in children with auditory neuropathy. Ear Hear 23:239–253
Miyamoto RT, Kirk KI, Renshaw J, et al (1999) Cochlear implantation in auditory neuropathy. Laryngoscope 109:181–185
Starr A, Sininger Y, Nguyen T, et al (2001) Cochlear receptor (microphonic and summating potentials, otoacoustic emissions) and auditory pathway (auditory brain stem potentials) activity in auditory neuropathy. Ear Hear 22:91–99
Ghiz AF, Salt AN, DeMott JE, et al (2001) Quantitative anatomy of the round window and cochlear aqueduct in guinea pigs. Hear Res 162:105–112
Patuzzi RB, Yates GK, Johnstone BM (1989) The origin of the low-frequency microphonic in the fi rst cochlear turn of guinea-pig. Hear Res 39:177–188
Dallos P (1973) The auditory periphery: biophysics and physiology. Academic Press, New York
Russell IJ, Sellick PM (1983) Low-frequency characteristics of intracellularly recorded receptor potentials in guinea-pig cochlear hair cells. J Physiol 338:179–206
Zheng XY, Ding DL, McFadden SL, et al (1997) Evidence that inner hair cells are the major source of cochlear summating potentials. Hear Res 113:76–88
Durrant JD, Wang J, Ding DL, et al (1998) Are inner or outer hair cells the source of summating potentials recorded from the round window? J Acoust Soc Am 104:370–376
Dolan DF, Xi L, Nuttall AL (1989) Characterization of an EPSP-like potential recorded remotely from the round window. J Acoust Soc Am 86:2167–2171
Sellick P, Patuzzi R, Robertson D (2003) Primary afferent and cochlear nucleus contributions to extracellular potentials during tone-bursts. Hear Res 176:42–58
Kiang NYS, Watanabe T, Thomas EC, et al (1965) Discharge patterns of single fi bres in the cat’s auditory nerve. Research Monograph no. 35. MIT Press, Cambridge
McMahon CM, Patuzzi RB (2002) The origin of the 900 Hz spectral peak in spontaneous and sound-evoked round-window electrical activity. Hear Res 173:134–152
Starr A, Sininger YS, Pratt H (2000) The varieties of auditory neuropathy. J Basic Clin Physiol Pharmacol 11:215–230
O’Leary SJ, Mitchell TE, Gibson WP, et al (2000) Abnormal positive potentials in round window electrocochleography. Am J Otol 21:813–818
Patuzzi R, Sellick PM (1983) A comparison between basilar membrane and inner hair cell receptor potential input-output functions in the guinea pig cochlea. J Acous Soc Am 74:1734–1741
Withnell R (2001) The cochlear microphonic as an indication of outer hair cell function. Ear Hear 22:75–77
Deltenre P, Mansbach AL, Bozet C, et al (1999) Auditory neuropathy with preserved cochlear microphonics and secondary loss of otoacoustic emissions. Audiology 38:187–195
RodrÃguez-Ballesteros M, del Castillo FJ, MartÃn Y, et al (2003) Auditory neuropathy in patients carrying mutations in the otoferlin gene (OTOF). Hum Mutat 22:451–456
Varga R, Kelley PM, Keats BJ, et al (2003) Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene. J Med Genet 40:45–50
Roux I, Safieddine S, Nouvian R, et al (2006) Otoferlin, defective in a human deafness form, is essential for exocytosis at the auditory ribbon synapse. Cell 127:277–289
Zidanic M, Brownell WE (1990) Fine structure of the intracochlear potential field. I. The silent current. Biophys J 57:1253–1268
Moser T, Beutner D (2000) Kinetics of exocytosis and endocytosis at the cochlear inner hair cell afferent synapse of the mouse. Proc Natl Acad Sci U S A 97:883–888
Liberman MC (1990) Effects of chronic cochlear de-efferentation on auditory-nerve response. Hear Res 49:209–223
Le Prell CG, Shore SE, Hughes LF, et al (2003) Disruption of lateral efferent pathways: functional changes in auditory evoked responses. J Assoc Res Otolaryngol 4:276–290
Katsuki Y, Yanagisawa K, Kanzaki J (1966) Tetraethylammonium and tetrodotoxin: effects on cochlear potentials. Science 151:1544–1545
Lin X (1997) Action potentials and underlying voltage-dependent currents studied in cultured spiral ganglion neurons of the postnatal gerbil. Hear Res 108:157–179
Schmiedt RA, Okamura HO, Lang H, et al (2002) Ouabain application to the round window of the gerbil cochlea: a model of auditory neuropathy and apoptosis. J Assoc Res Otolaryngol 3:223–233
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McMahon, C.M., Patuzzi, R.B., Gibson, W.P., Sanli, H. (2009). Identification of Different Subtypes of Auditory Neuropathy Using Electrocochleography. In: Kaga, K., Starr, A. (eds) Neuropathies of the Auditory and Vestibular Eighth Cranial Nerves. Springer, Tokyo. https://doi.org/10.1007/978-4-431-09433-3_3
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DOI: https://doi.org/10.1007/978-4-431-09433-3_3
Publisher Name: Springer, Tokyo
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